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Childhood hearing loss

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https://www.readbyqxmd.com/read/28750130/prevalence-of-hearing-loss-among-children-9-to-11-years-old-the-generation-r-study
#1
Carlijn M P le Clercq, Gijs van Ingen, Liesbet Ruytjens, André Goedegebure, Henriette A Moll, Hein Raat, Vincent W V Jaddoe, Rob J Baatenburg de Jong, Marc P van der Schroeff
Importance: Hearing loss (HL), a major cause of disability globally, negatively affects both personal and professional life. Objective: To describe the prevalence of sensorineural hearing loss (SNHL) among a population-based cohort of 9- to 11-year-old children, and to examine potential associations between purported risk factors and SNHL in early childhood. Design, Setting, and Participants: The study was among the general, nonclinical, pediatric community within the city of Rotterdam, the Netherlands, and was conducted between 2012 and 2015 as a cross-sectional assessment within the Generation R Study, a population-based longitudinal cohort study from fetal life until adulthood...
July 27, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28738350/unilateral-sensorineural-hearing-loss-medical-context-and-etiology
#2
Antoine Paul, Sandrine Marlin, Marine Parodi, Isabelle Rouillon, Joanne Guerlain, Véronique Pingault, Vincent Couloigner, Erea Noel Garabedian, Françoise Denoyelle, Natalie Loundon
OBJECTIVE: Unilateral sensorineural hearing loss (USNHL) is known to impact on school performance and social skills during childhood, but the etiologies remain unclear. The aim of this study was to assess various etiologies and to study the clinical contexts in this population. METHODS: The study is a retrospective review. Characteristics of hearing loss (HL), audiometric parameters, imaging, and genetic and medical contexts were analyzed. RESULTS: Eighty children were included...
July 22, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28737048/ototoxicity-monitoring-in-children-treated-with-platinum-chemotherapy
#3
Beth Brooks, Kristin Knight
OBJECTIVE: To review the prevalence, mechanisms, clinical presentation, risk factors and implications of platinum-induced ototoxicity in paediatric cancer patients based on published evidence, discuss options for monitoring hearing in young children during treatment and review long-term follow-up guidelines. DESIGN: Narrative literature review. RESULTS: Children treated with cisplatin are at high risk of hearing loss and early, accurate identification of ototoxicity is important for medical decision making and hearing rehabilitation...
July 24, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28724398/whole-genome-sequencing-identifies-a-novel-alms1-gene-mutation-in-two-chinese-siblings-with-alstr%C3%A3-m-syndrome
#4
Lin Yang, Zixiu Li, Mei Mei, Xiaomei Fan, Guodong Zhan, Huijun Wang, Guoying Huang, Mingbang Wang, Weidong Tian, Wenhao Zhou
BACKGROUND: Alström syndrome is a rare multi-systemic disorder with a broad spectrum of symptoms. This syndrome is characterized by childhood retinal degeneration; sensorineural hearing loss; obesity; type 2 diabetes mellitus; cardiomyopathy; systemic fibrosis; and pulmonary, hepatic, and renal failure. CASE PRESENTATION: A Chinese quartet family with two siblings predominantly affected by cone-rod dystrophy and short stature were recruited. The craniofacial dysmorphism and on-set age-of-cone-rod dystrophy in the proband showed a minor intrafamilial variability...
July 19, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28717752/challenges-of-treating-childhood-medulloblastoma-in-a-country-with-limited-resources-20-years-of-experience-at-a-single-tertiary-center-in-malaysia
#5
Revathi Rajagopal, Sayyidatul Abd-Ghafar, Dharmendra Ganesan, Anita Zarina Bustam Mainudin, Kum Thong Wong, Norlisah Ramli, Vida Jawin, Su Han Lum, Tsiao Yi Yap, Eric Bouffet, Ibrahim Qaddoumi, Shekhar Krishnan, Hany Ariffin, Wan Ariffin Abdullah
PURPOSE: Pediatric medulloblastoma (MB) treatment has evolved over the past few decades; however, treating children in countries with limited resources remains challenging. Until now, the literature regarding childhood MB in Malaysia has been nonexistent. Our objectives were to review the demographics and outcome of pediatric MB treated at the University Malaya Medical Center between January 1994 and December 2013 and describe the challenges encountered. METHODS: Fifty-one patients with childhood MB were seen at University Malaya Medical Center...
April 2017: Journal of Global Oncology
https://www.readbyqxmd.com/read/28706081/brief-stimulus-exposure-fully-remediates-temporal-processing-deficits-induced-by-early-hearing-loss
#6
David B Green, Michelle M Mattingly, Yi Ye, Jennifer D Gay, Merri J Rosen
In childhood, partial hearing loss can produce prolonged deficits in speech perception and temporal processing. Early therapeutic interventions targeting temporal processing may improve later speech-related outcomes, however. Gap detection is a measure of auditory temporal resolution that relies on auditory cortex (ACx), and early auditory deprivation alters intrinsic and synaptic properties in ACx. Thus, early deprivation should induce deficits in gap detection, which should be reflected in ACx gap sensitivity...
July 13, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28697000/evaluation-and-management-of-syndromic-congenital-hearing-loss
#7
Geoffrey Casazza, Jeremy D Meier
PURPOSE OF REVIEW: The purpose of this review is to review the evaluation and management of children with syndromic hearing loss. Specific syndromes and the impact of those syndromes on managing hearing loss will be discussed. RECENT FINDINGS: Improved molecular testing has increased the ability to identify syndromes-associated hearing loss. Accurate diagnosis of syndromic hearing loss can guide discussions regarding prognosis and appropriate management options for the hearing impairment...
July 10, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28689189/early-hearing-detection-and-vocabulary-of-children-with-hearing-loss
#8
Christine Yoshinaga-Itano, Allison L Sedey, Mallene Wiggin, Winnie Chung
BACKGROUND AND OBJECTIVES: To date, no studies have examined vocabulary outcomes of children meeting all 3 components of the Early Hearing Detection and Intervention (EHDI) guidelines (hearing screening by 1 month, diagnosis of hearing loss by 3 months, and intervention by 6 months of age). The primary purpose of the current study was to examine the impact of the current EHDI 1-3-6 policy on vocabulary outcomes across a wide geographic area. A secondary goal was to confirm the impact of other demographic variables previously reported to be related to language outcomes...
July 8, 2017: Pediatrics
https://www.readbyqxmd.com/read/28686376/-otoscopic-pitfall-the-congenital-cholesteatoma
#9
Karma Lambercy, Pauline Vinckenbosch, Céline Richard
The congenital cholesteatoma is a rare benign tumor whose diagnosis is mainly made during childhood. Otoscopic examination shows a white retrotympanic collection with or without hearing loss. In case of a late diagnosis, the disease will spread to the surrounding structures, leading to ossicules destruction, facial palsy, sensorineural hearing loss and vestibular dysfunction. Surgery is the therapeutic gold-standard, with different techniques chosen based on the cholesteatoma's extension. Patient should benefit from a long term medical and radiological (MRI) follow-up...
October 5, 2016: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28652589/congenital-deafness-is-associated-with-specific-somatosensory-deficits-in-adolescents
#10
Rabih Moshourab, Valérie Bégay, Christiane Wetzel, Jan Walcher, Steven Middleton, Manfred Gross, Gary R Lewin
Hearing and touch represent two distinct sensory systems that both rely on the transformation of mechanical force into electrical signals. Here we used a battery of quantitative sensory tests to probe touch, thermal and pain sensitivity in a young control population (14-20 years old) compared to age-matched individuals with congenital hearing loss. Sensory testing was performed on the dominant hand of 111 individuals with normal hearing and 36 with congenital hearing loss. Subjects with congenital deafness were characterized by significantly higher vibration detection thresholds at 10 Hz (2-fold increase, P < 0...
June 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28647553/morphosyntactic-correctness-of-written-language-production-in-adults-with-moderate-to-severe-congenital-hearing-loss
#11
Elke Huysmans, Jan de Jong, Joost M Festen, Martine M R Coene, S Theo Goverts
OBJECTIVE: To examine whether moderate to severe congenital hearing loss (MSCHL) leads to persistent morphosyntactic problems in the written language production of adults, as it does in their spoken language production. DESIGN: Samples of written language in Dutch were analysed for morphosyntactic correctness and syntactic complexity. STUDY SAMPLE: 20 adults with MSCHL and 10 adults with normal hearing (NH). RESULTS: Adults with MSCHL did not differ from adults with NH in the morphosyntactic correctness and syntactic complexity of their written utterances...
June 15, 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/28647130/fever-induced-paroxysmal-weakness-and-encephalopathy-a-new-phenotype-of-atp1a3-mutation
#12
Sho T Yano, Kenneth Silver, Richard Young, Suzanne D DeBrosse, Roseànne S Ebel, Kathryn J Swoboda, Gyula Acsadi
BACKGROUND: We identified a group of patients with ATP1A3 mutations at residue 756 who display a new phenotype, distinct from alternating hemiplegia of childhood, rapid-onset dystonia-parkinsonism, and cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndromes. METHODS: Four patients with c.2267G>A (R756H) mutations from two families and two patients with c.2267G>T (R756L) mutations from one family are described and compared with the previously reported patients with mutations resulting in R756H and R756C protein variants...
April 29, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28629849/analysis-of-archived-newborn-dried-blood-spots-dbs-identifies-congenital-cytomegalovirus-as-a-major-cause-of-unexplained-pediatric-sensorineural-hearing-loss
#13
Lucy Meyer, Bazak Sharon, Tina C Huang, Abby C Meyer, Kristin E Gravel, Lisa A Schimmenti, Elizabeth C Swanson, Hannah E Herd, Nelmary Hernandez-Alvarado, Kirsten R Coverstone, Mark McCann, Mark R Schleiss
PURPOSE: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. METHODS: Over a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study...
June 7, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28609304/suprameatal-cochlear-implantation-in-a-charge-patient-with-a-novel-chd7-variant-and-kallmann-syndrome-phenotype-a-case-report
#14
Akira Ganaha, Tetsuya Tono, Tadashi Kaname, Kumiko Yanagi, Teruyuki Higa, Shunsuke Kondo, Hiroyuki Maeda, Mikio Suzuki
OBJECTIVE: We present the clinical findings, technique of the suprameatal cochlear implantation, postoperative auditory results, and genetic analysis of the CHD7 gene. PATIENT: A 19-year-old Japanese woman was referred because of progressive hearing loss since early childhood. She had used verbal language for the main mode of communication until the age of 17. Examination revealed coloboma, heart defect, choanal atresia, genital hypoplasia, and deafness, which was diagnosed as CHARGE syndrome...
August 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28607211/consensus-statement-of-the-indian-academy-of-pediatrics-on-newborn-hearing-screening
#15
Abraham Paul, Chhaya Prasad, S S Kamath, Samir Dalwai, M K C Nair, Waheeda Pagarkar
JUSTIFICATION: Hearing impairment is one of the most critical sensory impairments with significant social and psychological consequences. Reported incidence of congenital hearing loss is 30 per 10,000 children. Evidence-based, standardized national guidelines are needed for professionals to screen for hearing impairment during the neonatal period. PROCESS: The meeting on formulation of national consensus guidelines on developmental disorders was organized by Indian Academy of Pediatrics in Mumbai, on 18th and 19th December, 2015...
June 4, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28602134/the-effects-of-early-auditory-based-intervention-on-adult-bilateral-cochlear-implant-outcomes
#16
Stacey R Lim
OBJECTIVES: The goal of this exploratory study was to determine the types of improvement that sequentially implanted auditory-verbal and auditory-oral adults with prelingual and childhood hearing loss received in bilateral listening conditions, compared to their best unilateral listening condition. METHODS: Five auditory-verbal adults and five auditory-oral adults were recruited for this study. Participants were seated in the center of a 6-loudspeaker array. BKB-SIN sentences were presented from 0° azimuth, while multi-talker babble was presented from various loudspeakers...
June 12, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/28594466/bleeding-is-not-the-main-clinical-issue-in-many-patients-with-inherited-thrombocytopaenias
#17
REVIEW
F Melazzini, C Zaninetti, C L Balduini
Bleeding diathesis has been considered for a long time the main clinical issue impacting the lives of patients affected by inherited thrombocytopaenias. However, the number of known inherited thrombocytopaenias greatly increased in recent years, and careful evaluation of hundreds of patients affected by these 'new' disorders revealed that most of them are at risk of developing additional life-threatening disorders during childhood or adult life. These additional disorders are usually more serious and dangerous than low platelet count...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28590156/hearing-loss-after-platinum-treatment-is-irreversible-in-noncranial-irradiated-childhood-cancer-survivors
#18
Eva Clemens, Andrica Ch de Vries, Antoinette Am Zehnhoff-Dinnesen, Wim Je Tissing, Jacqueline J Loonen, Saskia Fm Pluijm, Eline van Dulmen-den Broeder, Dorine Bresters, Birgitta Versluys, Leontien Cm Kremer, Helena J van der Pal, Sebastian Jccm Neggers, Martine van Grotel, Marry M van den Heuvel-Eibrink
Cisplatin and carboplatin are effective antineoplastic agents. They are also considered to be potentially highly ototoxic. To date, no long-term follow-up data from well-documented cohorts with substantial numbers of childhood cancer survivors (CCS) with platinum-related hearing loss are available. Therefore, in this study, we studied the reversibility of ototoxicity from discontinuation of treatment onwards in a national cohort of platinum-treated survivors with hearing loss at the end of cancer treatment...
June 7, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28573831/auditory-and-otologic-profile-of-alstr%C3%A3-m-syndrome-comprehensive-single-center-data-on-38-patients
#19
Spencer Lindsey, Carmen Brewer, Olga Stakhovskaya, Hung Jeffrey Kim, Chris Zalewski, Joy Bryant, Kelly A King, Jürgen K Naggert, William A Gahl, Jan D Marshall, Meral Gunay-Aygun
Alström syndrome (AS) is a rare autosomal recessive ciliopathy caused by mutations in the ALMS1 gene. Hallmark characteristics include childhood onset of severe retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, and cardiomyopathy. Here we comprehensively characterize the auditory and otologic manifestations in a prospective case series of 38 individuals, aged 1.7-37.9 years, with genetically confirmed AS. Hearing loss was preceded by retinal dystrophy in all cases, and had an average age of detection of 7...
August 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28522090/hearing-loss-mediates-executive-function-impairment-in-sleep-disordered-breathing
#20
Catherine M Hill, Romola S Bucks, Colin R Kennedy, Dawn Harrison, Annette Carroll, Nicolas Upton, Alexandra M Hogan
BACKGROUND: Sleep-disordered breathing (SDB) is often co-morbid with conductive hearing loss in early childhood due to a shared aetiology of adenotonsillar hypertrophy. Hearing loss is independently associated with impairment of executive function and behavioural difficulties. We hypothesised that these impairments in children with SDB may be mediated through hearing loss. METHODS: Fifty-eight children including 37 snorers awaiting adenotonsillectomy and 21 healthy non-snoring controls, aged 3-5 years, were assessed with pure tone audiometry, Strengths and Difficulties (SDQ), Behaviour Rating of Executive Function (BRIEF-P), and Childhood Middle Ear Disease and Hearing questionnaires...
June 2017: Sleep Medicine
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